Langerhans cell granulomatosis

Czech version

Authors: J. Homolka ^{1, 2}; I. Haškovcová ³
Authors‘ workplace: I. klinika tuberkulózy a respiračních nemocí 1. lékařské fakulty UK a VFN Praha, přednosta prof. MU Dr. Jiří Homolka, DrSc. ¹; Pneumologická klinika 1. lékařské fakulty UK a FTN Praha, přednosta prof. MU Dr. Jiří Homolka, DrSc. ²; Ústav patologie 1. lékařské fakulty UK a VFN Praha, přednosta prof. MU Dr. Ctibor Povýšil, DrSc. ³
Published in: Vnitř Lék 2010; 56(Supplementum 2): 74-75
Category: Langerhans cell histiocytosis and some other Hematology rare diseases

Overview

Langerhans cell granulomatosis is a rare disease of unknown aetiology and characterized by an infiltration of alveolar septa with activated Langerhans cells and eosinophylic granulocytes. Accumulation of these cells leads to a development of multiple cystic formations. The involvement of the lungs is usually present on its own, more rarely it is seen as part of a systemic disease involving skeleton or posterior pituitary. The disease prognosis in adult patients is usually good, 80% of patients achieve remission or stabilization of the disease either spontaneously or following a corticosteroid therapy, 10 –⁠ 20% of cases progress to respiratory failure.

Key words:
Langerhans cell granulomatosis –⁠ diagnostics –⁠ BAL –⁠ treatment

Sources

1. Homolka J, Křepelka J, Velenská Z. Záměna histiocytózy X za kryptogenní fibrotizující alveolitidu. Prakt Lék 1991; 71 : 20 –⁠ 22.

2. Kodet R, Zítková M. Morfologické a rentgenové plicní nálezy při disseminované histiocytóze X. Čs Pediat 1985; 40 : 634 –⁠ 638.

3. Moore AD, Godwin JD, Müller NL et al. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology 1989; 172 : 249 –⁠ 254.

4. Skácel Z, Marel M, Vraštilová P et al. Histiocytóza z Langerhansových buněk. Stud Pneumol Phthiseol 2000; 60 : 150 –⁠ 156.

5. Tazi A. Adult pulmonary Langerhans’ cell histiocytosis. Eur Respir J 2006; 27 : 1272 –⁠ 1285.

6. Yağci B, Varan A, Cağlar M et al. Langerhans cell histiocytosis: retrospective analysis of 217 cases in a single center. Pediatr Hematol Oncol 2008; 25 : 399 –⁠ 408.

7. Zeppa P, Cozzolino I, Russo M et al. Pulmonary Langerhans cell histiocytosis (histiocytosis X) on bronchoalveolar lavage: a report of 2 cases. Acta Cytol 2007; 51 : 480 –⁠ 482.